目的观察揿针配合感觉统合训练治疗共济失调型脑性瘫痪的临床疗效。方法将105例共济失调型脑性瘫痪患者随机分为对照A组、对照B组和联合组,每组35例。对照A组采取揿针治疗,对照B组采用感觉统合训练治疗,联合组采用揿针配合感觉统合训练...目的观察揿针配合感觉统合训练治疗共济失调型脑性瘫痪的临床疗效。方法将105例共济失调型脑性瘫痪患者随机分为对照A组、对照B组和联合组,每组35例。对照A组采取揿针治疗,对照B组采用感觉统合训练治疗,联合组采用揿针配合感觉统合训练治疗。观察3组治疗前后粗大运动功能测试量表-88(gross motor function measure-88,GMFM-88)各功能区(A~E区)评分、步态各项指标(步长、步速、步宽)、平衡控制能力各项指标(躯干前后标准差、躯干左右标准差及躯干总标准差)、表面肌电信号各项指标[内收肌及腓肠肌积分肌电值(integrated electromyography,IEMG)、表面肌电均方根值(root mean square,RMS)]及儿科生活质量量表(the pediatric quality of life inventory,PedsQL)各项评分(生理功能、情感功能、社交功能)的变化情况,比较3组临床疗效。结果3组治疗前后GMFM-88各功能区评分、步态各项指标、平衡控制能力各项指标、表面肌电信号各项指标及PedsQL各项评分组内比较,差异具有统计学意义(P<0.01)。联合组治疗后GMFM-88各功能区评分及步长、步速均明显高于对照A组和对照B组,步宽及表面肌电信号各项指标均明显低于对照A组和对照B组,差异均具有统计学意义(P<0.05)。联合组及对照B组治疗后平衡控制能力各项指标均明显低于对照A组,PedsQL各项评分均明显高于对照A组,差异均具有统计学意义(P<0.05)。联合组总有效率为94.3%,显著高于对照A组的74.3%和对照B组的68.6%,差异均具有统计学意义(P<0.05)。结论揿针配合感觉统合训练治疗共济失调型脑性瘫痪疗效确切,可增强核心肌群稳定性,改善患者步态,提高粗大运动及平衡能力。展开更多
We report the case of a 33- year-old woman with limited systemic sclerosis and chronic progressive sensory ataxic neuropathy. Sural nerve biopsy showed loss of myelinated fibers mostly those of large diameter, axonal ...We report the case of a 33- year-old woman with limited systemic sclerosis and chronic progressive sensory ataxic neuropathy. Sural nerve biopsy showed loss of myelinated fibers mostly those of large diameter, axonal degeneration and infiltration of macrophages, but no signs of vasculitis. Physical examination, laboratory testing, neurophysiological and neuroradiological examinations suggested that the dorsal root was primarily affected in this patient. Cytokine analysis by multiplex bead array assay revealed that IL- 1β and GM-CSF were increased both in serum and CSF. Although her symptoms did not respond to corticosteroid therapy, intravenous immunoglobulin (IVIg) therapy resulted in marked improvement. IVIg could be effective in case of immune-mediated reversible neuronal dysfunction associated with collagen disease without vasculitis.展开更多
The authors describe siblings with progressive external ophthalmoplegia (PEO) due to a novel heterozygous A to G transition at nucleotide 955 of C10Orf2 (Twinkle). The mutation was not identified in parents’ blood, h...The authors describe siblings with progressive external ophthalmoplegia (PEO) due to a novel heterozygous A to G transition at nucleotide 955 of C10Orf2 (Twinkle). The mutation was not identified in parents’ blood, hair follicles, buccal mucosa, or urinary epithelium, indicating germ line mosaicism. One sibling presented with sensory ataxic neuropathy, dysarthria, and ophthalmoparesis (SANDO), a phenotype previously associated with the POLG1 gene, highlighting the clinical overlap in autosomal PEO.展开更多
文摘目的观察揿针配合感觉统合训练治疗共济失调型脑性瘫痪的临床疗效。方法将105例共济失调型脑性瘫痪患者随机分为对照A组、对照B组和联合组,每组35例。对照A组采取揿针治疗,对照B组采用感觉统合训练治疗,联合组采用揿针配合感觉统合训练治疗。观察3组治疗前后粗大运动功能测试量表-88(gross motor function measure-88,GMFM-88)各功能区(A~E区)评分、步态各项指标(步长、步速、步宽)、平衡控制能力各项指标(躯干前后标准差、躯干左右标准差及躯干总标准差)、表面肌电信号各项指标[内收肌及腓肠肌积分肌电值(integrated electromyography,IEMG)、表面肌电均方根值(root mean square,RMS)]及儿科生活质量量表(the pediatric quality of life inventory,PedsQL)各项评分(生理功能、情感功能、社交功能)的变化情况,比较3组临床疗效。结果3组治疗前后GMFM-88各功能区评分、步态各项指标、平衡控制能力各项指标、表面肌电信号各项指标及PedsQL各项评分组内比较,差异具有统计学意义(P<0.01)。联合组治疗后GMFM-88各功能区评分及步长、步速均明显高于对照A组和对照B组,步宽及表面肌电信号各项指标均明显低于对照A组和对照B组,差异均具有统计学意义(P<0.05)。联合组及对照B组治疗后平衡控制能力各项指标均明显低于对照A组,PedsQL各项评分均明显高于对照A组,差异均具有统计学意义(P<0.05)。联合组总有效率为94.3%,显著高于对照A组的74.3%和对照B组的68.6%,差异均具有统计学意义(P<0.05)。结论揿针配合感觉统合训练治疗共济失调型脑性瘫痪疗效确切,可增强核心肌群稳定性,改善患者步态,提高粗大运动及平衡能力。
文摘We report the case of a 33- year-old woman with limited systemic sclerosis and chronic progressive sensory ataxic neuropathy. Sural nerve biopsy showed loss of myelinated fibers mostly those of large diameter, axonal degeneration and infiltration of macrophages, but no signs of vasculitis. Physical examination, laboratory testing, neurophysiological and neuroradiological examinations suggested that the dorsal root was primarily affected in this patient. Cytokine analysis by multiplex bead array assay revealed that IL- 1β and GM-CSF were increased both in serum and CSF. Although her symptoms did not respond to corticosteroid therapy, intravenous immunoglobulin (IVIg) therapy resulted in marked improvement. IVIg could be effective in case of immune-mediated reversible neuronal dysfunction associated with collagen disease without vasculitis.
文摘The authors describe siblings with progressive external ophthalmoplegia (PEO) due to a novel heterozygous A to G transition at nucleotide 955 of C10Orf2 (Twinkle). The mutation was not identified in parents’ blood, hair follicles, buccal mucosa, or urinary epithelium, indicating germ line mosaicism. One sibling presented with sensory ataxic neuropathy, dysarthria, and ophthalmoparesis (SANDO), a phenotype previously associated with the POLG1 gene, highlighting the clinical overlap in autosomal PEO.
文摘目的:建立一成功率高,感觉症状确实的感觉性神经元神经病的动物模型。并同时观察不同剂量维生素 B6对于大鼠感觉性神经系统的作用。方法:50只雌性Wistar大鼠分别给予600mg／kg(1周或2周),400mg／kg(4周), 200mg／kg(4周或8周)与100mg／kg(4周或8周)维生素B6,腹腔注射,每天一次。取其后根神经节(DRG),周围神经及脊髓作光镜与电镜分析。染色方法有HE,Luxol Fast Blue,Masson三色与银浸染色。半薄切片用甲苯胺兰染色,超薄切片用醋酸双氧铀与枸橼酸铅染色。结果:腰段DRG受累最重,其次为颈段、胸段。大剂量组导致感觉性神经元神经病,动物步态不稳,甚至瘫痪。DRG细胞体体积减少或坏死,伴以轴突萎缩与崩解,并可见吞噬细胞吞噬现象。小剂量组动物无异常临床表现,DRG神经元病变轻微,但存在轴突萎缩与变性。电镜下发现近端突与胞体均有细胞骨架异常。脊髓后束中薄束比楔束病变重。结论:多种因素包括用药时间、用药剂量及不同亚群神经元的药物敏感性不同,均可影响维生素B6神经毒性的最终表现。
